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C1q Nephropathy in a 2-Year-Old Boy: A Case Report

Received: 4 January 2023    Accepted: 27 January 2023    Published: 9 February 2023
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Abstract

Background: C1q nephropathy occurs when C1q is significantly deposited in the mesangial region and systemic lupus erythematosus can be excluded as a diagnosis. Clinical Description: A case of a 2-year-old boy who was diagnosed with steroid-resistant C1q nephropathy. As he was initially managed as a case of idiopathic nephrotic syndrome, prednisolone was administered. However, as his condition did not improve with steroid treatment, other causes for nephrotic were considered, including infections. As it was initially refractory to steroid treatment, a cytomegalovirus infection was strongly considered. Based on the histological findings, C1q deposition was noted in the mesangium, which confirmed the diagnosis of C1q nephropathy. Cyclosporine A was initiated, which resulted in complete remission from the disease. When administering high doses of steroids, it is necessary to recognize the risk of infection. Conclusion: C1q nephropathy is considered to be a heterogeneous disease group from the clinicopathological point of view. In order to treat established C1q nephropathy, it is necessary to clarify the pathological significance of C1q deposition in glomeruli. As it was initially refractory to steroid treatment, CMV infection was strongly considered. When administering high doses of steroids, it is necessary to recognize the risk of infection.

Published in American Journal of Pediatrics (Volume 9, Issue 1)
DOI 10.11648/j.ajp.20230901.13
Page(s) 15-19
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

C1q Nephropathy, CMV Infection, Children

References
[1] Jennette JC, Hipp CG. Immunohistopathologic evaluation of C1q in 800 renal biopsy specimens. Am J Clin Pathol. 1985; 83: 415-420.
[2] Jennette JC, Hipp CG. C1q nephropathy: a distinct pathologic entity usually causing nephrotic syndrome. Am J Kidney Dis. 1985; 6: 103-110.
[3] Markowitz GS, Schwimmer JA, Stokes MB, et al. C1q nephropathy: a variant of focal segmental glomerulosclerosis. Kidney Int. 2003; 64: 1232-1240.
[4] Lau KK, Gaber LW, Delos Santos NM, et al. C1q nephropathy: features at presentation and outcomes. Pediatr Nepfrol. 2005; 20: 744-749.
[5] Levart TK, Kenda RB, Cavic MA, et al. C1q nephropathy in children. Pediatr Nephrol. 2005; 20: 1756-1761.
[6] Iskandar SS, Browning MC, Lorentz WB. A pediatric clinicopathologic study. Am J Kidney Dis. 1991; 18: 459-465.
[7] Fukuma Y, Hisano S, Segawa Y, et al. Clinicopathologic correlation of C1q nephropathy in children. Am J Kidney Dis. 2006; 47: 412-418.
[8] Nishida M, Hamaoka K, Fujimoto S. A clinicopathological study of C1q nephropathy Japanese Journal of Pediatric Nephrology. 2014; 27 (1): 1-5.
[9] Scott E, Rita DS, Michael CB. C1q nephropathy in the pediatric population: pathology and pathogenesis. Pediatr Nephrol. 2010; 25: 1385-1396.
[10] Fukuma Y, Hisano S, Segawa Y, Niimi K, Tsuru N, Kaku Y, Hatae K, Kiyoshi Y, Mitsudome A, Iwasaki H: Clinicopathologic correlation of C1q nephropathy in children. Am J Kidney Dis 2006; 47: 412–418.
[11] Hisano S, Fukuma Y, Segawa Y, et al. Clinicopathologic correlation and outcome of C1q nephropathy. Clin J Am Soc Nephrol. 2008; 3: 1637-1643.
[12] Mii A, Shimizu A, Masuda Y, et al. Current status and issues of C1q nephropathy. Clin Exp Nephrol. 2009; 13: 263-74.
[13] Kuwano M, Ito Y, Amamoto Y, Aida K. A case of congenital nephrotic syndrome associated with positive C1q immunofluorescence. Pediatr Nephrol. 1993; 7: 452-454.
[14] So H, Hatae K, Onoyama S, et al. C1q nephropathy with steroid resistant nephrotic syndrome in an 1-year-old girl. Japanese Journal of Pediatric Nephrology. 2007; 20 (2): 108-112.
[15] Ginzler E, Diamond H, Kaplan D, et. al. Computer analysis of factors influencing frequency of infection in systemic lupus erythematosus. Arthritis Rheum. 1978; 21 (1): 37-44.
Cite This Article
  • APA Style

    Reika Omura, Hiroshi Tamura, Keishiro Furuie, Shohei Kuraoka, Hitoshi x Kuraoka. (2023). C1q Nephropathy in a 2-Year-Old Boy: A Case Report. American Journal of Pediatrics, 9(1), 15-19. https://doi.org/10.11648/j.ajp.20230901.13

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    ACS Style

    Reika Omura; Hiroshi Tamura; Keishiro Furuie; Shohei Kuraoka; Hitoshi x Kuraoka. C1q Nephropathy in a 2-Year-Old Boy: A Case Report. Am. J. Pediatr. 2023, 9(1), 15-19. doi: 10.11648/j.ajp.20230901.13

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    AMA Style

    Reika Omura, Hiroshi Tamura, Keishiro Furuie, Shohei Kuraoka, Hitoshi x Kuraoka. C1q Nephropathy in a 2-Year-Old Boy: A Case Report. Am J Pediatr. 2023;9(1):15-19. doi: 10.11648/j.ajp.20230901.13

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  • @article{10.11648/j.ajp.20230901.13,
      author = {Reika Omura and Hiroshi Tamura and Keishiro Furuie and Shohei Kuraoka and Hitoshi x Kuraoka},
      title = {C1q Nephropathy in a 2-Year-Old Boy: A Case Report},
      journal = {American Journal of Pediatrics},
      volume = {9},
      number = {1},
      pages = {15-19},
      doi = {10.11648/j.ajp.20230901.13},
      url = {https://doi.org/10.11648/j.ajp.20230901.13},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajp.20230901.13},
      abstract = {Background: C1q nephropathy occurs when C1q is significantly deposited in the mesangial region and systemic lupus erythematosus can be excluded as a diagnosis. Clinical Description: A case of a 2-year-old boy who was diagnosed with steroid-resistant C1q nephropathy. As he was initially managed as a case of idiopathic nephrotic syndrome, prednisolone was administered. However, as his condition did not improve with steroid treatment, other causes for nephrotic were considered, including infections. As it was initially refractory to steroid treatment, a cytomegalovirus infection was strongly considered. Based on the histological findings, C1q deposition was noted in the mesangium, which confirmed the diagnosis of C1q nephropathy. Cyclosporine A was initiated, which resulted in complete remission from the disease. When administering high doses of steroids, it is necessary to recognize the risk of infection. Conclusion: C1q nephropathy is considered to be a heterogeneous disease group from the clinicopathological point of view. In order to treat established C1q nephropathy, it is necessary to clarify the pathological significance of C1q deposition in glomeruli. As it was initially refractory to steroid treatment, CMV infection was strongly considered. When administering high doses of steroids, it is necessary to recognize the risk of infection.},
     year = {2023}
    }
    

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  • TY  - JOUR
    T1  - C1q Nephropathy in a 2-Year-Old Boy: A Case Report
    AU  - Reika Omura
    AU  - Hiroshi Tamura
    AU  - Keishiro Furuie
    AU  - Shohei Kuraoka
    AU  - Hitoshi x Kuraoka
    Y1  - 2023/02/09
    PY  - 2023
    N1  - https://doi.org/10.11648/j.ajp.20230901.13
    DO  - 10.11648/j.ajp.20230901.13
    T2  - American Journal of Pediatrics
    JF  - American Journal of Pediatrics
    JO  - American Journal of Pediatrics
    SP  - 15
    EP  - 19
    PB  - Science Publishing Group
    SN  - 2472-0909
    UR  - https://doi.org/10.11648/j.ajp.20230901.13
    AB  - Background: C1q nephropathy occurs when C1q is significantly deposited in the mesangial region and systemic lupus erythematosus can be excluded as a diagnosis. Clinical Description: A case of a 2-year-old boy who was diagnosed with steroid-resistant C1q nephropathy. As he was initially managed as a case of idiopathic nephrotic syndrome, prednisolone was administered. However, as his condition did not improve with steroid treatment, other causes for nephrotic were considered, including infections. As it was initially refractory to steroid treatment, a cytomegalovirus infection was strongly considered. Based on the histological findings, C1q deposition was noted in the mesangium, which confirmed the diagnosis of C1q nephropathy. Cyclosporine A was initiated, which resulted in complete remission from the disease. When administering high doses of steroids, it is necessary to recognize the risk of infection. Conclusion: C1q nephropathy is considered to be a heterogeneous disease group from the clinicopathological point of view. In order to treat established C1q nephropathy, it is necessary to clarify the pathological significance of C1q deposition in glomeruli. As it was initially refractory to steroid treatment, CMV infection was strongly considered. When administering high doses of steroids, it is necessary to recognize the risk of infection.
    VL  - 9
    IS  - 1
    ER  - 

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Author Information
  • Department of Pediatrics, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan

  • Department of Pediatrics, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan

  • Department of Pediatrics, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan

  • Department of Pediatrics, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan

  • Department of Pediatrics, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan

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